10. Acute lupus pneumonitis
• Non specific
• May simulate infections
• 1-4 % of patients
• Cough, dyspnoea, pleuritic
pain, hypoxaemia, and fever
• CXR: diffuse acinar infiltrates( u/l as well as
b/l)
• Sterile sputum/ ET cultures
11. • Histologicaly:
alveolar wall damage and necrosis,
inflammatory cell infiltration,
oedema, haemorrhage,hyaline membrane
• CT Scan:
alveolitis (a ground glass appearance)
fibrosis (a honey comb appearance)
12. • BAL: lymphocytic predominance
sterile cultures
• Gallium scintigraphy: increased uptake
• DLCO : decreased
• Video-assisted thoracoscopic biopsy or open
lung biopsy: last option
15. Treatment:
• Broad spectrum antibiotic cover
• Systemic prednisone (1 to 1.5 mg/kg per day
in divided doses)
• Intravenous pulse glucocorticoids (1 gram of
methylprednisolone/ day for 3days)
immunosuppressive drugs
(cyclophosphamide)
16. Sick patients( tachypnea, hypoxemia)
Patients with no response after 72 hours of
prednisone
• PROGNOSIS:
Fulminant course
High mortality
Poor prog: BAL with eosinophils, postpartum
17. CXR showing right lower zone
shadows
Improvement of CXR after 4
weeks of therapy
21. Chronic interstitial pneumonia in a 35 year old woman
with SLE.HRCT scan shows extensive ground glass
opacities admixed with coarse linear bands and
honeycomb cysts.
22. • TREATMENT: depends upon inflammatory or
fibrotic pattern
• Dual therapy
• High glucocorticoids (prednisone 1 to 2
mg/kg/day) and cyclophosphamide
• Transition to either azathioprine or
mycophenolate mofetil after 6 to 12 months.
• Nothing established for fibrotic disease
• PIRFENIDONE( tyrosine kinase inhibitor): trial
basis
23. PULMONARY HYPERTENSION
• Rare complication
• 5-10% of patients
• Increases with age
• 2 % have right heart failure
• Dyspnea
• Chest pain
• Chronic non productive cough
24. • Prominent JVP
• Hepatomegaly
• Ascites
• Peripheral edema
• CXR: enlarged pulmonary arteries with clear
lung fields.
straightening of the left heart border and
attenuation of the peripheral vessels
26. • ECG: RVH
• PFT: restrictive pattern
decreased DLCO
• 2 D ECHO: inc PAP and TR ( tricuspid valve
insufficiency)
• CT CHEST: dilatation of main pulmonary artery
and heterogenesity of lung perfusion
28. SHRINKING LUNG SYNDROME
• Less common manifestation
• Dyspnea,
• Pleuritic chest pain
• Progressive decrease in lung volume
• No evidence of interstitial fibrosis or pleural
disease on chest CT
• myositis or myopathy affecting both
diaphragms
29. • DIAGNOSIS: triad of
dyspnea
clear chest x-rays
elevated diaphragms
• TREATMENT: alone or combination of
Glucocorticoids,
Theophylline
Immunosuppressive therapy
32. • BAL: bloody fluid
hemosiderin-laden macrophages
HPE: capillaritis
immune complex deposition
bland hemorrhage
HIGH PROBABILITY: active lupus
high titre of ds DNA antibodies
lupus nephritis
34. • TREATMENT: one or combination of
high dose steroids
cyclophosphamide
• Plasmapheresis:
severe alveolar haemorrhage refractory
to corticosteroids and cytotoxic agents
Lupus 1997;6:730–3
35. Cryptogenic organizing pneumonia
(COP)
• BOOP
• Plugs of fibrous tissue in bronchioles and
alveolar ducts
• Dry cough
• CXR: multiple infiltrates
• HRCT: dense consolidation
• Treatment: oral prednisone(1 mg/Kg/day)
Ann Rheum Dis 1991; 50:956
36. Pulmonary venoocclusive disease
(PVOD)
• Rare cause of PHTN
• Dyspnea and hypoxemia
• Intimal fibrosis---- occlusion of pulmonary
veins
• HRCT CHEST: thickened interlobular septa
lymph node enlargement
ground glass opacities